Vulvar cancer - Wikipedia, the free encyclopedia

23/03/15 00:27

Vulvar cancer
From Wikipedia, the free encyclopedia

Vulvar cancer is a malignant, invasive growth in the

vulva, or the outer portion of the female genitals, that
typically affects older women. The disease accounts for
only 0.6% of cancer diagnoses[1] but 5% of gynecologic
cancers in the United States.[2] The labia majora are the
most common site involved representing about 50% of
all cases, followed by the labia minora.[3] The clitoris
and Bartholin glands may rarely be involved.[4]

Vulvar cancer
Classification and external resources
ICD-9 184.4
(http://www.icd9data.com/getICD9Code.ashx?
icd9=184.4)
NCI

Vulvar cancer
(http://www.cancer.gov/cancertopics/types/vulvar)

Vulvar cancer is separate from vulvar intraepithelial neoplasia (VIN), a superficial lesion of the epithelium that
has not invaded the basement membrane - or a pre-cancer.[5] VIN may progress to carcinoma-in-situ and,
eventually, squamous cell cancer.
According to the American Cancer Society, in 2014, there were about 4,850 new cases of vulvar cancer and
1,030 deaths from the disease.[6] In the United States, five-year survival rates for vulvar cancer are around
70%.[7]

Contents
1 Types
1.1 Squamous cell carcinoma
1.2 Melanoma
1.3 Basal cell carcinoma
1.4 Other lesions
2 Signs and Symptoms
3 Causes
4 Diagnosis
4.1 Differential diagnosis
4.2 Staging
5 Treatment
6 Prognosis
7 Epidemiology
8 References

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9 External links

Types
Squamous cell carcinoma
Most vulvar cancer (approximately 90%)[8] is squamous cell carcinoma, which originates from epidermal
squamous cells, the most common type of skin cell. Carcinoma-in-situ is a precursor lesion of squamous cell
cancer that does not invade through the basement membrane. While this type of lesion is more common with
older age, young women with risk factors may also be affected. In the elderly, complications may occur due to
the presence of other medical conditions.
Squamous lesions tend to arise in a single site and occur most commonly in the vestibule.[9] They grow by local
extension and spread via the local lymph system. The lymphatics of the labia drain to the upper vulva and mons,
then to both superficial and deep inguinal and femoral lymph nodes. The last deep femoral node is called the
Cloquets node.[9] Spread beyond this node reaches the lymph nodes of the pelvis. The tumor may also invade
nearby organs such as the vagina, urethra, and rectum and spread via their lymphatics.
A verrucous carcinoma of the vulva is a rare subtype of squamous cell cancer and tends to appear as a slowly
growing wart. Verrucous vulvar cancers tend to have good overall prognoses.[10]

Melanoma
Melanoma is the second most common type of vulvar cancer and causes 8-10% of vulvar cancer cases.[9] These
lesions arise from melanocytes, the cells that give skin color and are most common in Caucasian women 50-80
years old.[11] Melanoma of the vulva behaves like melanoma in any other location and may affect a much
younger population.
There are three distinct types of vulvar melanoma: superficial spreading, nodular, and acral lentigous
melanoma. Vulvar melanomas are unique in that they are microstaged with the Chung, Clark and/or Breslow
systems, which specify stage and tumor depth of invasion. In general, they come with a high risk of metastasis
and carry a poor overall prognosis.[9]

Basal cell carcinoma

Basal cell carcinoma makes up about 1-2% of vulvar cancer. These tend to be slow-growing lesions on the labia
majora but can occur anywhere on the vulva. Their behavior is similar to basal cell cancers in other locations.
They often grow locally and have low risk for deep invasion or metastasis.
Treatment involves excision, but these lesions have a tendency to recur if not completely removed.[12]

Other lesions
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Other lesions, such as adenocarcinoma (of the Bartholin glands, for example) or sarcoma, may cause vulvar
cancer as well.

Signs and Symptoms

Typically, a lesion presents in the form of a lump or ulcer and may be associated with itching, irritation, local
bleeding or discharge, in addition to pain with urination or pain during sexual intercourse. Due to modesty or
embarrassment, patients may put off seeing a doctor.
Melanomas tend to display the typical asymmetry, uneven borders and dark discoloration.
Adenocarcinoma can arise from the Bartholin gland and present with a painful lump.

Causes
The cause of vulvar cancer is unclear; however, some conditions such as lichen sclerosus, squamous dysplasia
or chronic vulvar itching may precede cancer. In younger women affected with vulvar cancer, risk factors
include low socioeconomic status, human papillomavirus (HPV) infection, multiple sexual partners, cigarette
use and cervical cancer.[9] Patients that are infected with HIV tend to be more susceptible to vulvar cancer as
well.

Diagnosis
Examination of the vulva is part of the gynecologic evaluation and should include a thorough inspection of the
perineum, including areas around the clitoris and urethra, and palpation of the Bartholin's glands.[13] The exam
may reveal an ulceration, lump or mass in the vulvar region. Any suspicious lesions need to be sampled, or
biopsied. This can generally be done in an office setting under local anesthesia. Small lesions can be removed
under local anesthesia as well. Additional evaluation may include a chest X-ray, an intravenous pyelogram,
cystoscopy or proctoscopy, as well as blood counts and metabolic assessment.

Differential diagnosis
Other cancerous lesions in the differential diagnosis include Paget's disease of the vulva and vulvar
intraepithelial neoplasia (VIN). Non-cancerous vulvar diseases include lichen sclerosus, squamous cell
hyperplasia, and vulvar vestibulitis. A number of diseases cause infectious lesions including herpes genitalis,
human papillomavirus, syphilis, chancroid, granuloma inguinale, and lymphogranuloma venereum.

Staging
Anatomical staging supplemented preclinical staging starting in 1988. FIGOs revised TNM classification
system uses tumor size (T), lymph node involvement (N) and presence or absence of metastasis (M) as criteria
for staging. Stages I and II describe the early stages of vulvar cancer that still appear to be confined to the site of
origin. Stage III cancers include greater disease extension to neighboring tissues and inguinal lymph nodes on

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one side. Stage IV indicates metastatic disease to inguinal nodes on both sides or distant metastases.[14]
Illustrations showing stages of vulvar cancer (http://oncolex.org/en/Gynecological-cancer/Diagnoses/Vulvarcancer/Background/Staging)

Stage 1A and 1B vulvar

cancer

Stage 2 vulvar cancer

Stage 3 vulvar cancer

Stage 4A vulvar cancer

Stage 4B vulvar cancer

Treatment
Staging and treatment are generally handled by an oncologist familiar with gynecologic cancer. Surgery is a
mainstay of therapy depending on anatomical staging and is usually reserved for cancers that have not spread
beyond the vulva.[14] Surgery may involve a wide local excision, radical partial vulvectomy, or radical complete
vulvectomy with removal of vulvar tissue, inguinal and femoral lymph nodes. In cases of early vulvar cancer,
the surgery may be less extensive and consist of wide excision or a simple vulvectomy. Surgery is significantly
more extensive when the cancer has spread to nearby organs such as the urethra, vagina, or rectum.
Complications of surgery include wound infection, sexual dysfunction, edema and thrombosis, as well as
lymphedema secondary to dissected lymph nodes.[15]
Sentinel lymph node (SLN) dissection is the identification of the main lymph node(s) draining the tumor, with
the aim of removing as few nodes as possible, decreasing the risk of adverse effects. Location of the sentinel
node(s) may require the use of technetium(99m)-labeled nano-colloid, or a combination of technetium and 1%
isosulfan blue dye, wherein the combination may reduce the number of women with "'missed"' groin node
metastases compared with technetium only.[15]

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Radiation therapy may be used in more advanced vulvar cancer cases

when disease has spread to the lymph nodes and/or pelvis. It may be
performed before or after surgery. Chemotherapy is not usually used as
primary treatment but may be used in advanced cases with spread to the
bones, liver or lungs. It may also be given at a lower dose together with
radiation therapy.[16]
Women with vulvar cancer should have routine follow-up and exams
with their oncologist, often every 3 months for the first 2-3 years after
treatment. They should not have routine surveillance imaging to monitor
the cancer unless new symptoms appear or tumor markers begin

Diagram of the incisions made in a

vulvectomy, a treatment for vulvar
cancer.

rising.[17] Imaging without these indications is discouraged because it is

unlikely to detect a recurrence or improve survival and is associated with its own side effects and financial
costs.[17]

Prognosis
Overall, five-year survival rates for vulvar cancer are around 78%[9] but may be affected by individual factors
including cancer stage, cancer type, patient age and general medical health. Five-year survival is greater than
90% for patients with stage I lesions but decreases to 20% when pelvic lymph nodes are involved. Lymph node
involvement is the most important predictor of prognosis.[18] Thus, early diagnosis is important.

Epidemiology
Vulvar cancer causes less than 1% of all cancer cases and deaths but around 6% of all gynecologic cancers
diagnosed in the UK. Around 1,200 women were diagnosed with the disease in 2011, and 400 women died in
2012.[19]

References
1. "American Cancer Society: What are the key statistics about vulvar cancer?"
(http://www.cancer.org/cancer/vulvarcancer/detailedguide/vulvar-cancer-key-statistics). www.cancer.org. Retrieved
6 November 2014.
2. "National Cancer Institute- General Information About Vulvar Cancer"
(http://www.cancer.gov/cancertopics/pdq/treatment/vulvar/HealthProfessional#Reference1.1). National Cancer Institute
at the National Institutes of Health. 3/12/2014. Retrieved 11/6/14. Check date values in: |date=, |accessdate= (help)
3. "National Cancer Institute- General Information About Vulvar Cancer"
(http://www.cancer.gov/cancertopics/pdq/treatment/vulvar/HealthProfessional#Reference1.1). National Cancer Institute
at the National Institutes of Health. 3/12/2014. Retrieved 11/6/2014. Check date values in: |date=, |accessdate=
(help)
4. www.cancer.org http://www.cancer.org/cancer/vulvarcancer/detailedguide/vulvar-cancer-what-is-vulvar-cancer.

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Retrieved 11/6/2014. Check date values in: |accessdate= (help); Missing or empty |title= (help)
5. "What is Vulvar Cancer?" (https://www.sgo.org/patients-caregivers-survivors/caregivers/vulvar-cancer-generalinformation/). Society of Gynecologic Oncology. Retrieved 19 November 2014.
6. "American Cancer Society" (http://www.cancer.org/acs/groups/content/@research/documents/webcontent/acspc042151.pdf). Retrieved 30 April 2014.
7. "SEER Stat Fact Sheets: Vulvar Cancer" (http://seer.cancer.gov/statfacts/html/vulva.html). NCI. Retrieved 18 June 2014.
8. "Vulvar Cancer - October 1, 2002 - American Family Physician" (http://www.aafp.org/afp/2002/1001/p1269.html).
Retrieved 2010-03-06.
9. Hoffman, Barbara; Schorge, John; Schaffer, Joseph; Halvorson, Lisa; Bradshaw, Karen; Cunningham, Gary (2012).
Williams Gynecology (2nd ed.). The McGraw-Hill Company, Inc. ISBN 978-0-07-171672-7.
10. "American Cancer Society: What is Vulvar Cancer?" (http://www.cancer.org/cancer/vulvarcancer/detailedguide/vulvarcancer-what-is-vulvar-cancer). www.cancer.org. Retrieved 11/6/2014. Check date values in: |accessdate= (help)
11. Evans, RA (1994). "Review and current perspectives of cutaneous malignant melanoma.". Journal of the American
College of Surgeons. PMID 7952494 (https://www.ncbi.nlm.nih.gov/pubmed/7952494).
12. DeCherney, Alan H.; Nathan, Lauren; Laufer, Neri; Roman, Ashley S. (2013). Current Diagnosis and Treatment:
Obstetrics and Gynecology (11th ed.). USA: The McGraw-Hill Companies, Inc. ISBN 978-0-07-163856-2.
13. "Vulvar Cancer" (http://www.health.am/cr/vulvar-cancer/). Gynecologic Neoplasms. Armenian Health Network,
Health.am. 2005. Retrieved 2007-11-08.
14. International Federation of Gynecologists and Obstetricians (FIGO) (2000). "Staging classification and clinical practice
guidelines of gynaecologic cancers"
(http://web.archive.org/web/20060423064053/http://www.igcs.org/guidelines/guideline_staging-booklet.pdf). Archived
from the original (http://www.igcs.org/guidelines/guideline_staging-booklet.pdf) on 2006-04-23. Retrieved 2006-10-13.
15. Lawrie, Theresa A; Patel, Amit; Martin-Hirsch, Pierre PL; Bryant, Andrew; Ratnavelu, Nithya DG; Naik, Raj; Ralte,
Angela; Patel, Amit (2014). "Sentinel node assessment for diagnosis of groin lymph node involvement in vulval cancer".
doi:10.1002/14651858.CD010409.pub2 (https://dx.doi.org/10.1002%2F14651858.CD010409.pub2).
16. "What are the treatment options?" (https://www.sgo.org/patients-caregivers-survivors/patients/vulvar-cancer-what-arethe-treatment-options/). Society of Gynecologic Oncology. Retrieved 19 November 2014.
17. Society of Gynecologic Oncology (February 2014), "Five Things Physicians and Patients Should Question"
(http://www.choosingwisely.org/doctor-patient-lists/society-of-gynecologic-oncology/), Choosing Wisely: an initiative of
the ABIM Foundation (Society of Gynecologic Oncology), retrieved 19 February 2013
18. Farias-Eisner, R; Cirisano, FD; Grouse, D (1994). "Conservative and individualized surgery for early squamous
carcinoma of the vulva: the treatment of choice for stage I and II (T12N01M0) disease". Gynecologic Oncology 53:
55-58. PMID 8175023 (https://www.ncbi.nlm.nih.gov/pubmed/8175023).
19. "Vulval cancer statistics" (http://www.cancerresearchuk.org/cancer-info/cancerstats/types/vulva/). Cancer Research UK.
Retrieved 28 October 2014.

External links
Guide by the (http://www.cancer.org/docroot/CRI/CRI_2_3x.asp?dt=45) American Cancer Society
Information from the (http://www.cancer.gov/cancertopics/pdq/treatment/vulvar/healthprofessional)
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National Cancer Institute

Vulvar Cancer. Timothy P. Canavan and Donna Cohen. (http://www.aafp.org/afp/20021001/1269.html)
Am Fam Physician 2002;66:1269-74,1276. Review article for general practitioners.
Cancer.Net: Vulvar Cancer (http://www.cancer.net/cancer-types/vulvar-cancer)
Retrieved from "http://en.wikipedia.org/w/index.php?title=Vulvar_cancer&oldid=651257911"
Categories: Gynaecological cancer Papillomavirus-associated diseases
This page was last modified on 13 March 2015, at 22:46.
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